• Molecules and Cells
• /
• v.39 no.10
• /
• pp.722-727
• /
• 2016

Cardiomyopathy is a major cause of death worldwide. Based on pathohistological abnormalities and clinical manifestation, cardiomyopathies are categorized into several groups: hypertrophic, dilated, restricted, arrhythmogenic right ventricular, and unclassified. Dilated cardiomyopathy, which is characterized by dilation of the left ventricle and systolic dysfunction, is the most severe and prevalent form of cardiomyopathy and usually requires heart transplantation. Its etiology remains unclear. Recent genetic studies of single gene mutations have provided significant insights into the complex processes of cardiac dysfunction. To date, over 40 genes have been demonstrated to contribute to dilated cardiomyopathy. With advances in genetic screening techniques, novel genes associated with this disease are continuously being identified. The respective gene products can be classified into several functional groups such as sarcomere proteins, structural proteins, ion channels, and nuclear envelope proteins. Nuclear envelope proteins are emerging as potential molecular targets in dilated cardiomyopathy. Because they are not directly associated with contractile force generation and transmission, the molecular pathways through which these proteins cause cardiac muscle disorder remain unclear. However, nuclear envelope proteins are involved in many essential cellular processes. Therefore, integrating apparently distinct cellular processes is of great interest in elucidating the etiology of dilated cardiomyopathy. In this mini review, we summarize the genetic factors associated with dilated cardiomyopathy and discuss their cellular functions.

• Proceedings of the Korean Nuclear Society Conference
• /
• 2005.10a
• /
• pp.610-611
• /
• 2005

• The Journal of Korean Medicine
• /
• v.18 no.1
• /
• pp.430-445
• /
• 1997

In order to know the effects of Calculus $Bovis{\cdot}F디$ Ursi aqua-acupuncture ($C{\cdot}F$ aqua-acupuncture) to the liver damage, we carried out the treatment of $C{\cdot}F$ aqua-acupuncture and injection of Kam-Du-Tang on the liver damaged rat which is induced by aconitine, and then ultrastructural study has been to the liver tissue. The results were as follows: 1. In the hepatic cell of normal group, the nuclear envelope is round and electro-density of nucleoplasm is relatively low, and have one or two large nucleolus. Cell organelle is developed rough endoplasmic reticulum(RER) and many mitochondria, glycogen granules, smooth endoplasmic reticulum(SER) and Golgi apparatus. 2. In 24 hours control group, nuclear envelope is very irregular, RER and most of cell organell is shown that destroyed by aconitine. This is similar to the 48 hours control group. In 72 hours control group, RER is recovered somewhat but cristae of mitochondria is not seen the shape 3.In 24 hours $C{\cdot}F$ aqua-acupuncture group, nuclear envelope is very irregular and cristernae is very dilatable and many mitochondria is dilationed and the cristae is not definite like the control group. In 48 hours $C{\cdot}F$ aqua-acupuncture group, nuclear envelope is relatively round and chromatin is regular. Cristernae of RER and ribosme is somewhat imperfect but is similar the normal group, and the cristae of mitchondria is shown definitely like the normal group. In 72 hours $C{\cdot}F$ aqua-acupuncture group, nuclear envelope is round and cell organell is similar to normal group. 4. In 24 hours Kam-Du-Tang group, nuclear envlope is very irregular and cell organelles are destroyed by the effects of aconitine. In 48 hours Kam-Du-Tang group, nuclear envelope is somewhat irregular but chromatin is relatively regular. RER is seperated regularly through the cytoplasm, but cannot make the structure of lamina, and cistennae is very dilationed. many of glycogen granules is seperated regularly. In 72 hours Kam-Du-Tang group, RER makes the lamina. Mitochondria is a little but inner space is dilationed so the shape of is not definite and a lot of glycogen granules are accumulated in several places. It see the above result, hepatic cell that is damaged by aconitine is effected chiefly by the $C{\cdot}F$ acupuncture and effect of acupuncture is similar to the detoxicated effect of Kam-Du-Tang.

• Korean Journal of Plant Tissue Culture
• /
• v.27 no.5
• /
• pp.407-409
• /
• 2000

Outer envelope membrane proteins of chloroplasts encoded by the nuclear genome are transported without the N-terminal transit peptide. Here, we investigated the targeting mechanism of AtOEP7, an Arabidopsis homolog of small outer envelope membrane proteins in vivo. AtOEP7 was expressed transiently in protoplasts or stably in transgenic plants as fusion proteins with GFP. In both cases AtOEP7:GFP was targeted to the outer envelope membrane when assayed under a fluorescent microscope or by Western blot analysis. Except the transmembrane domain, deletions of the N- or C-terminal regions of AtOEP7 did not affect targeting although a region closed to the C-terminal side of the transmembrane domain affected the targeting efficiency. Targeting experiments with various hybrid transmembrane mutants revealed that the amino acid sequence of the transmembrane domain determines the targeting specificity The targeting mechanism was further studied using a fusion protein, AtOEP7：NLS：GFP, that had a nuclear localization signal. AtOEP7：NLS：GFP was efficiently targeted to the chloroplast envelope despite the presence of the nuclear localization signal. Taken together, these results suggest that the transmembrane domain of AtOEP7 functions as the sole determinant of targeting specificity and that AtOEP7 may be associated with a cytosolic component during translocation to the chloroplast envelope membrane.

• Proceedings of the Korean Nuclear Society Conference
• /
• 2004.05a
• /
• pp.302.1-302.1
• /
• 2004

• Nuclear Engineering and Technology
• /
• v.55 no.5
• /
• pp.1687-1707
• /
• 2023

This paper presents a molten salt reactor (MSR) design parameter sensitivity study using a nodal dynamic modelling methodology with explicitly modified point kinetics equation and Mann's model for heat transfer. Six parameters that can impact MSR safety are evaluated. A MATLAB-Simulink model inspired by Thorcon's 550MWth MSR is used for parameter evaluations. A safety envelope was formed to encapsulate power, maximum and minimum temperature, and temperature-induced reactivity feedback. The parameters are perturbed by ±30%. The parameters were then ranked by their subsequent impact on the considered safety envelope, which ranks acceptable parameter uncertainty. The model is openly available on GitHub.

• Proceedings of the Korean Nuclear Society Conference
• /
• 2002.10a
• /
• pp.420.2-420
• /
• 2002

• Korean Journal Plant Pathology
• /
• v.2 no.1
• /
• pp.1-11
• /
• 1986

Negatively stained dip preparations from Euonymus showing vein clear symptoms revealed bacilliform particles. The particles tentatively referred to as the Euonymus vein clear virus(EVCV) have a relatively complex structure, measuring 230-280nm in length and 70-80nm in diameter. They have an envelope, 8-10nm thick, provided with evenly spaced beadlike projection about 5-6nm long. The inner tubular core which had no envelope showed helical structures, 200-220nm long, and 50-55nm in diameter. This inner tubular core is interpreted as the virus nucleocapsid. A striking association of virus particles with the nuclei of infected cells was apparent from sections which showed numberous virus particles at the nuclear periphery and in what appeared to be intranuclear virus particle inclusions. Careful examination of these apparent inclusions revealed the presence of the nuclear envelope surrounding them, in addition to cytoplasmic organelles within them. Such profiles were interpreted as having arisen when the sections passed through invaginations of the cytoplasm into the nucleus. In all the sections showing virus particles associated with the nucleus, large number of virus particles were found to be present in expanded areas between the two lamellae of the nuclear envelope. This location is suggested as a possible site of virus assembly. Serveal micrographs of particles found in this location suggested incorporation of the inner lamella of the nuclear envelope into the viral envelope. Various micrographs indicated a possible helical arrangement of certain components present in the virus core.

• Molecules and Cells
• /
• v.39 no.9
• /
• pp.669-679
• /
• 2016

N-acetyl-D-glucosamine kinase (GlcNAc kinase or NAGK) primarily catalyzes phosphoryl transfer to GlcNAc during amino sugar metabolism. Recently, it was shown NAGK interacts with dynein light chain roadblock type 1 (DYNLRB1) and upregulates axo-dendritic growth, which is an enzyme activity-independent, non-canonical structural role. The authors examined the distributions of NAGK and NAGK-dynein complexes during the cell cycle in HEK293T cells. NAGK was expressed throughout different stages of cell division and immunocytochemistry (ICC) showed NAGK was localized at nuclear envelope, spindle microtubules (MTs), and kinetochores (KTs). A proximity ligation assay (PLA) for NAGK and DYNLRB1 revealed NAGK-dynein complex on nuclear envelopes in prophase cells and on chromosomes in metaphase cells. NAGK-DYNLRB1 PLA followed by Lis1/NudE1 immunostaining showed NAGK-dynein complexes were colocalized with Lis1 and NudE1 signals, and PLA for NAGK-Lis1 showed similar signal patterns, suggesting a functional link between NAGK and dynein-Lis1 complex. Subsequently, NAGK-dynein complexes were found in KTs and on nuclear membranes where KTs were marked with CENP-B ICC and nuclear membrane with lamin ICC. Furthermore, knockdown of NAGK by small hairpin (sh) RNA was found to delay cell division. These results indicate that the NAGK-dynein interaction with the involvements of Lis1 and NudE1 plays an important role in prophase nuclear envelope breakdown (NEB) and metaphase MT-KT attachment during eukaryotic cell division.

• Nuclear Engineering and Technology
• /
• v.55 no.3
• /
• pp.968-980
• /
• 2023

The dynamic structural responses are sensitive to the time-frequency content of seismic waves, and seismic input motions in time-history analysis are usually required to be compatible with design response spectra according to nuclear codes. In order to generate spectra-compatible input motions while maintaining the intrinsic non-stationarity of seismic waves, an improved time-domain approach is proposed in this paper. To maintain the nonstationary characteristics of the given seismic waves, a new time-frequency envelope function is constructed using the Hilbert amplitude spectrum. Based on the intrinsic mode functions (IMFs) obtained from given seismic waves through variational mode decomposition, a new corrective time history is constructed to locally modify the given seismic waves. The proposed corrective time history and time-frequency envelope function are unique for each earthquake records as they are extracted from the given seismic waves. In addition, a dimension reduction iterative technique is presented herein to simultaneously superimpose corrective time histories of all the damping ratios at a specific frequency in the time domain according to optimal weights, which are found by the genetic algorithm (GA). Examples are presented to show the capability of the proposed approach in generating spectra-compatible time histories, especially in maintaining the nonstationary characteristics of seismic records. And numerical results reveal that the modified time histories generated by the proposed method can obtain similar dynamic behaviors of AP1000 nuclear power plant with the natural seismic records. Thus, the proposed method can be efficiently used in the design practices.